Effects of Empowerment Program on the Burden of Care in Mothers of Children with Phenylketonuria

Objectives Phenylketonuria (PKU) is a genetic disease of children that need a lifelong diet for its treatment. Because of the high burden of care, parents and particularly mothers are prone to mental disorders or psychiatric adverse reactions. We aimed to investigate the effect of empowerment of mothers on the burden of caring for children with PKU. Materials & Methods This was a semi-experimental (before-after with control group) study. Overall, 50 mothers of children with PKU that were referred to Endocrine Clinic in Qods Hospital of Qazvin City, northwestern Iran in 2016, were selected and randomly divided into intervention and control groups. Data collection was made by using demographic characteristics of mothers and children and also the Zarit burden interview questionnaires. Empowerment program was delivered during nine sessions of holistic and family oriented empowerment model in the intervention group. The control group received routine services. Data were analyzed using descriptive and inferential statistics through SPSS software. Results In intervention group, the mean score of overall care burden and its dimensions in the mothers of children with PKU in post-test was significantly lower than the pre-test (41.20 ± 5.04 vs. 58.24 ± 3.96; P<0.001); but pre-test and post-test scores in the control group had no significant difference (58.4 ± 5.22 vs. 58.96 ± 4; P=0.327). In other words empowerment program was able to reduce the burden of caring in mothers. Conclusion: Empowerment program can reduce the burden of care in mothers of children with PKU. Thus empowerment training is necessary for this group of mothers in the form of workshops and educational pamphlets.


Introduction
Phenylketonuria (PKU) is a genetic disorder caused by a deficiency of the phenylalanine hydroxylase enzyme (1, 2). If the disease does not recognize early in life or the affected children with PKU do not be compliant with treatment regimen (food), it leads to severe cognitive or behavioral problems, seizures and autistic symptoms (3). The incidence of PKU is 1 case per 10000 live births (4). Despite studies that took place in recent years on families with PKU in Iran, due to the lack of a generalized screening and registering system, exact extent of the disease is unclear in Iran, however, the rate of PKU in Iran is about 1 in 6000 (5).
Caring for a child with an incurable disease has negative impact on parents. Parents of children with chronic diseases have vulnerable personality because they think that they are responsible for their child's condition or are unable to properly care for him. In some cases, the identity of the family may be disintegrated and parents may show psychological disorders (6). Similarly, parents of children with PKU may feel guilty for transmission of affected gen to their children, so they blame themselves (7). Caring for these patients, have a burden of care for parents. In most cases, control of phenylalanine level, maintaining accurate and balanced diet and handling, occupational and speech therapy programs are assigned to mothers (5). Because of the burden of care, many of them may show some degree of psychological reactions including emotional distress, confusion, anger, frustration, impatience, crying, obsession that leads to disturbed sleep and appetite, anxiety and depression (8,9). "Burden of care is a concept emerging in the literature that describes the physical, emotional, social, and financial problems experienced by family caregivers" (10).
Burden refers to the impact that induced from caring process of a sick person who is unable to perform his/her Activities of Daily Living (ADL).
This impact affected the physical, financial, psychological, and social aspects of life of a caregiver (11). Care burden is a term frequently used for caregivers who provide informal care, that is, they do not have an educational background in the health field and are not paid for providing such care (11). Burden of care refers to the negative reactions that this person's experience (12). It occurs because of lack of balance between the needs and times spent on caring and other duties and also social, personal, physical and emotional roles of careers (13). Frequently, burden of care is more defined by its impacts and consequences on caregivers. It has both subjective and objective aspects. Subjective aspects refers to degree of impression that caregivers feel and its objective aspects show effects of burden on the family life like as performing of daily tasks (14).
Empowerment is a nursing intervention and educational model that helps mothers to feel the desired change (15,16). "Empowerment paradigm involves a fundamental redefinition of roles and relationships of health care professionals and patients" (16). Family empowerment can lead to increase the life expectancy and increasing quality of life in patients with common genetic diseases such as thalassemia, hemophilia or PKU (17). Family-centered empowerment programs emphasize that family has an effective role on the motivation, psychological, knowledge, attitudes and perceived threat of the members and its primary goal is to empower family system that can lead to health promotion (18). So far family

Material & Methods
This was a semi-experimental study, before-after with control group and was performed on 50 mothers of children with PKU that referred to the Outpatient Clinic of Qods Hospital of Qazvin City north-west of Iran in 2016.
Purposeful convenient sampling was used. The inclusion criteria were medical diagnosis of PKU in children, the age of the children ≤12 yr, living in Qazvin Province, caregiver willingness and cooperation to participate in the study. Exclusion criteria were parental refusal to cooperate with the research. By considering the 95% confidence interval, α=5% and 80% test power, the estimated sample size was 50 mothers that randomly assigned to intervention (25) and control (25)

Results
Sample was formed from 50 mothers of children with PKU. The average age of mothers in the intervention group (35.7 ± 5.6 yr) and control (36.1 ± 6.4 yr) and also other demographic characteristics in two groups were not significantly different (Table 2).   (Table 3).  All authors agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.